Evaluation of coexisting optic nerve head drusen and glaucoma with optical coherence tomography

OBJECTIVE: Optic nerve head drusen often make evaluation of the nerve head difficult to interpret. In addition, visual field defects are known to occur in patients with optic disk drusen, resembling glaucomatous damage. The authors report two cases of coincident optic nerve head drusen and glaucoma, in which the use of optical coherence tomography (OCT) in evaluating the nerve fiber layer was beneficial. PARTICIPANTS: Two patients with both optic nerve head drusen and glaucoma, one with primary open angle glaucoma, the other with pseudoexfoliation glaucoma were evaluated. Both patients had asymmetric optic disk drusen, with clinically visible drusen only in one eye. INTERVENTION: Ophthalmologic examination, color and red-free photography, automated Humphrey visual field testing and OCT were performed. RESULTS: Nerve fiber layer loss as measured by OCT was found to be greater than expected by the appearance of the optic nerve head and red-free photography, with visual fields consistent with findings in case 1. In case 2, visual fields were full, despite nerve fiber layer thinning seen by OCT and red-free photography. CONCLUSIONS: There can be significant nerve fiber layer thinning in patients with both glaucoma and optic disk drusen, despite the appearance of the optic nerve head in these patients. The cup margin may be obscured by the drusen, giving rise to a falsely full-appearing disk. In such cases, OCT may provide a useful means to quantitatively measure the nerve fiber layer thickness and to aid in the management of these patients by detecting nerve fiber layer thinning earlier than would otherwise be possible.     

Retinal ganglion cell layer and visual function in a patient with optic disc drusen

BACKGROUND: To correlate the retinal ganglion cell pattern to visual acuity and visual field data in a patient with bilateral optic disc drusen, a quantitative clinicopathological study was carried out. METHODS: Both retinae of a patient with optic drusen were whole-mounted. Retinal ganglion cell counts were made using a sampling scheme covering the whole retina and compared to the findings in 10 normal retinae. Relative ganglion cell reduction in the drusen retinae was correlated to clinical data. RESULTS: The total retinal ganglion cell count was reduced from 1244858+/-98736 in normal retinae to 305319 on the right and 527571 on the left eye with optic disc drusen. Large ganglion cells had a better chance of survival. Parafoveal ganglion cell loss was 57% for the right and 36% for the left eye, while visual acuity was 0.8 and 1.0 respectively. The mean light sensitivity loss increased from the centre (6.2 dB) to paracentral (9.9 dB), mid-peripheral (13.7 dB) and outer peripheral (15.0 dB) retina, while ganglion cell losses were smallest in outer peripheral retina (21.9%), followed by central (53.0%), mid-peripheral (70.9%) and paracentral retina (87.7%). CONCLUSION: These data validate Frisén's theory on central retinal resolution and provide the structural basis for the clinical rule that low visual acuity should not be attributed to disc drusen. Automated light sense perimetry gives an inadequate picture of retinal damage caused by optic disc drusen.     

Synthesis of the selective 5-hydroxytryptamine 4 (5-HT4) receptor agonist (+)-(S)-2-chloro-5-methoxy-4-[5-(2-piperidylmethyl)-1,2,4-oxadiazol-3-y l]aniline

A 52-year-old white woman was first diagnosed with a tumor of the right optic nerve in 1972. She remained asymptomatic until 1992, when she had a seizure on the left side of her body from a frontoparietal glioblastoma multiforme. Ophthalmic examination revealed enlargement of the eye tumor. This case provides clinical documentation spanning 20 years of a growing, pigmented tumor of the optic nerve head shown histopathologically to be a retinal pigment epithelial adenoma.     

Diabetic papillopathy: two case reports in individuals with adult onset diabetes mellitus

BACKGROUND: Diabetic papillopathy is a benign unilateral or bilateral optic neuropathy with transient optic disk edema and minimal reduction in visual function. The optic disk edema typically resolves in a few months with no resulting optic atrophy and minimal or no decrease in acuity. The exact etiology of the disk edema is unknown, but theories include retinal vascular leakage into and surrounding the optic nerve and disruption of axoplasmic flow resulting from microvascular disease of the optic nerve head vasculature. CASE REPORTS: Two adult patients receiving insulin for type II diabetes mellitus manifested bilateral disk edema and minimal visual dysfunction. Both patients showed funduscopic evidence of mild-to-moderate nonproliferative diabetic retinopathy O.D. and O.S., and one patient had clinically significant macular edema in both eyes. The diagnosis in both cases was diabetic papillopathy. Both patients had significant resolution of their disk edema in 3 to 6 months, with stable acuities and no signs of optic atrophy. CONCLUSIONS: Although diabetic papillopathy is a well-known clinical entity in patients with type I diabetes, the clinical profile can be expanded to include individuals with type II diabetes.     

Effect of strength training on EMG of human skeletal muscle

An 8-year-old white girl with a history of vertigo, nausea, and vomiting developed a progressive hearing loss, bilateral retinal arteriolar narrowing in each eye, vasoproliferation, and subsequent intravitreal hemorrhage. An attempt at peripheral retinal ablation with cryotherapy in the left eye resulted in retinal detachment. Spontaneous retinal detachment occurred in the right eye and was successfully repaired. Repeated intermittent hemorrhages occurred despite intraocular diathermy. Three years after onset, visual acuity was R.E.: 6/21 (20/66) and L.E.: light perception. She remains totally deaf. A 20-year-old white woman developed severe bilateral sensorineural hearing loss with poorly functioning labyrinths, followed by midperipheral retinal arteriolar occlusions and vasoproliferation on the optic nerve head. Progressive retinal neovascularization was followed by rubeosis iridis and repeated episodes of intravitreal bleeding. Six years after onset, visula acuity was R.E.: hand motions, and L.E.: 6/3 (20/100). She remains totally deaf. Both patients were of normal gestation, development, and mentality, without evidence of other systemic disease. The cause of this disease was not found.     

Effects of 0.5% apraclonidine on optic nerve head and peripapillary retinal blood flow

AIMS: To examine the effects of 0.5% apraclonidine on optic nerve head (ONH) and peripapillary retinal blood flow by scanning laser Doppler flowmetry (SLDF). METHODS: ONH and peripapillary retinal blood flow of 17 healthy subjects were measured by SLDF before and 1 hour and 3 hours after unilateral administration of 0.5% apraclonidine. The fellow eyes were treated with balanced salt solution and the examiners were masked as to which eye was treated with apraclonidine. On each occasion, three scans were obtained and haemodynamic variables (volume, flow, and velocity) were analysed at eight locations, four in the neural rim and four in the peripapillary retina, avoiding ophthalmoscopically visible vessels. The statistical significance of changes from the baseline value of variables and the differences in the measured quantities between apraclonidine treated eyes and fellow eyes at each time point were evaluated using Wilcoxon signed rank test. RESULTS: The intraocular pressure was reduced significantly in apraclonidine treated eyes by 15.0% (p = 0.001) at 1 hour and 30.0% (p = 0.000) at 3 hours after administration. In the volume, flow, or velocity of ONH and peripapillary retinal blood flow, there were no significant changes from the baseline values at 1 hour and 3 hours after apraclonidine administration in either apraclonidine treated eyes (p > 0.4) or fellow eyes (p > 0.2). Also, no significant differences were found in the measured quantities between apraclonidine treated eyes and fellow eyes at each time point (p > 0.1). CONCLUSION: A single dose of topical apraclonidine 0.5% in healthy subjects does not have adverse effects on the ONH and peripapillary retinal blood flow.     

Change in the land vertebrate community under new conditions in nature]

PURPOSE: To report bilateral congenital optic nerve head pits in monozygotic siblings. METHOD: Case reports. RESULTS: Pits were found in abnormally large optic disks in both eyes of two otherwise healthy female monozygotic siblings aged 15 years. Pit size increased and visual acuity decreased with increased optic disk area. In one eye, nonrhegmatogenous retinal detachment developed that eventually necessitated pars plana vitrectomy. The siblings' parents were unremarkable. CONCLUSIONS: Congenital optic nerve head pits can occur bilaterally in otherwise healthy monozygotic siblings with ophthalmologically unremarkable parents. Associated nonrhegmatogenous retinal detachment may be treated by pars plana vitrectomy. Pit size is positively correlated with disk area.     

Bacillary angiomatosis associated with myositis in a patient infected with human immunodeficiency virus

PURPOSE: To report a patient with optic nerve avulsion caused by forceful rotation of the globe that occurred when his thumb penetrated the orbit while he was diving. METHODS: A 17-year-old boy was initially examined for sudden loss of vision after jumping feet first from a bridge 50 feet above a river. Upon hitting the water, he felt his right thumb push into his right globe. The patient underwent ophthalmologic and imaging examination. RESULT: Examination disclosed a tear of the optic nerve head from the sclera temporally in the right eye. CONCLUSION: Optic nerve avulsion occurs secondary to forceful rotation of the eye with tearing of the optic nerve as it exits the globe.     

High levels of serum allopregnanolone in women with premature ovarian failure

BACKGROUND: Blue-on-yellow (B/Y) perimetry can reveal visual field defects earlier and larger in extent than white-on-white (W/W) perimetry. The Heidelberg Retina Tomograph (HRT) produces a three-dimensional image of the optic disc. The aim of this study was to compare the strength of the association of the B/Y and W/W visual hemifield mean deviation (HMD) variables with the optic nerve head (ONH) morphological variables of the respective area. METHODS: We evaluated one randomly chosen eye of 40 normal subjects and 37 patients with ocular hypertension and different stages of glaucoma. The B/Y and W/W visual fields (program 30-2) were obtained with a Humphrey perimeter. Results of both visual fields were adjusted for the patient's age and lens transmission index measured with a lens fluorometer. HMD was calculated as the difference between the measured and expected hemifield mean sensitivity values, predicted by the regression model fitted in our nonglaucomatous subject data. The HRT with the software version 1.11 was used to acquire and evaluate the topographic measurements of the optic disc. RESULTS: The B/Y and W/W visual field HMDs showed statistically significant correlation with ONH parameters such as cup shape measure (CSM), rim volume, rim area, mean retinal nerve fiber layer (RNFL) thickness and RNFL cross-sectional area. With forward stepwise logistic regression analysis using B/Y hemifield data 38% of the glaucoma patient's normal W/W hemifields were classified abnormal. With the CSM alone in the model 52% of the cases were classified abnormal. CONCLUSIONS: B/Y visual field hemifield mean deviation values correlate well with ONH parameters examined with the HRT.     

Neuro-ophthalmology, for the pediatrician

The pediatrician needs to develop some skills in evaluating afferent visual functions and ocular motor abnormalities. He must know some fundamental neuro-ophthalmologic facts to prevent his patients from undergoing unnecessary diagnostic and surgical procedures. In addition, he needs to understand the fundamentals of strabismus and amblyopia, which are briefly considered in this article but are explored thoroughly elsewhere in this issue of PEDIATRIC ANNALS. First, the most common type of nystagmus in children is congenital nystagmus. These children often have a head turn or tilt. Also, it should be remembered that numerous drugs may cause nystagmus. Second, any child with a head turn or tilt must be considered to have some ocular motor abnormality until a complete ophthalmologic evaluation has eliminated this possibility. In addition, before a child is considered to have an isolated sixth-nerve palsy, Duane's retraction syndrome should be looked for. Also, before an inferior oblique palsy is diagnosed, a Brown's tendon sheath syndrome should be considered. Thus, any ocular muscle abnormality deserves an ophthalmologic evaluation. Third, when bilateral swollen optic nerves are noted as an incidental finding, drusen of the optic nerve head should be suspected. A unilateral swollen disk with decreased visual acuity and a Marcus Gunn pupil should bring to mind a papillitis, which is a local inflammatory condition of the optic nerve head. Remember, papilledema is a bilateral condition secondary to increased intracranial pressure with normal vision. Children with papilledema usually have other signs of increased intracranial pressure. If the article has convinced you of only two facts -- that head tilts and turns are usually ocular in origin, and that bilaterally swollen optic nerves in an otherwise asymptomatic child may be optic nerve drusen -- it has accomplished its purpose.     

Optic nerve damage following argon laser photocoagulation in a human eye

Argon laser photocoagulation of diabetic neovascular formations overlying the optic disc risks injury to the nerve fibers. Isolated reports of postoperative field defects have appeared but extensive pathologic studies of the effects on nerve fibers are lacking. In an eye with a malignant melanoma nasally, laser photocoagulation was directed at the superior temporal artery on the disc. Applications were made repeatedly until spasm was produced at two sites. The following day, the eye was enucleated. Histopathologic examination revealed loss of endothelial cells and absence of nuclei in the media of the treated artery, and coagulative necrosis of nerve fibers around the vessel. Extensive damage to the peripapillary outer segments and retinal pigment epithelium also was demonstrated.     

Effects of topical nipradilol, a beta-blocking agent with alpha-blocking and nitroglycerin-like activities, on aqueous humor dynamics and fundus circulation

PURPOSE: To study the effects of nipradilol, a nonselective beta-blocker with alpha 1-blocking activity and nitroglycerin-like activity, on aqueous humor dynamics and optic nerve head (ONH) circulation in albino rabbits. METHODS: Experiments were carried out during the dark phase, in conscious rabbits conditioned to a schedule of alternating 12-hour periods of light and dark. The blood-aqueous barrier permeability and the aqueous flow rate were determined fluorophotometrically. The effect on outflow to general blood circulation and uveoscleral outflow were determined by using the fluorophotometric Diamox technique, and the effect on the uveoscleral outflow was further assessed by using the anterior chamber perfusion method. The ONH circulation was estimated by using the laser speckle method. RESULTS: Unilateral topical administration of 0.25% nipradilol solution lowered intraocular pressure (IOP) with relatively weak contralateral effects in a dose-dependent manner with a maximum reduction of 6 mm Hg and an effect duration of 6 hours. Twice-daily instillation for 14 days showed no attenuation of the effects. Single instillation of 0.25% nipradilol showed no significant effect on blood-aqueous barrier permeability and decreased aqueous flow rate in the treated eye (17%; P < 0.01) and in the contralateral eye (9%, P < 0.05). Nipradilol produced no significant effect on outflow facility to general blood circulation, whereas it substantially increased uveoscleral outflow. Twice-daily 0.25% nipradilol instillation increased ONH tissue blood velocity by 13% (P < 0.01), which was probably attributable to locally penetrating drug. CONCLUSIONS: Because of its ability to lower IOP and to increase uveoscleral outflow and optic nerve head circulation in rabbits, further studies are warranted to determine whether nipradilol has potential as an antiglaucoma agent in humans.     

Neuronal death in glaucoma

Glaucoma is recognized to have its major detrimental effect upon the eye by killing retinal ganglion cells. The process of cell death appears to be initiated at the optic nerve head, though other sites of injury are possible but unsubstantiated. At present the injury at the nerve head seems related to the level of the eye pressure, but its detailed mechanism is as yet unexplained. There is a greater loss of ganglion cells from some areas of the eye, and this feature of glaucoma seems related to the regional structure of the supporting connective tissues of the optic nerve head. Larger retinal ganglion cells have been consistently shown to have somewhat greater susceptibility to injury in glaucoma, though all cells are injured, even early in the process. Ganglion cells die by apoptosis in human and experimental glaucoma, opening several potential areas for future therapies to protect them from dying. Neurotrophin deprivation is one possible cause of cell death and replacement therapy is a potential approach to treatment.     

Evaluation of the retinal nerve fiber layer

In normal eyes, the retinal nerve fiber layer (RNFL) is usually best visible in the inferior temporal part of the fundus, followed by the superior temporal region, the nasal superior region and the nasal inferior region. This distribution correlates with the configuration of the neuroretinal rim, the diameter of the retinal arterioles, the location of the foveola, and the lamina cribrosa morphology. With increasing age, the RNFL visibility decreases diffusely without preferring special fundus regions and without the development of localized defects. With all optic nerve diseases, the visibility of the RNFL is decreased in addition to the age-related loss, in a diffuse and/or a localized manner. The localized defects are wedge-shaped and not spindle-like defects, running toward or touching the optic disk border. Typically occurring in about 20% of all glaucoma eyes, they can be found also in other ocular diseases, such as optic disk drusen, toxoplasmotic retinochoroidal scars, longstanding papilledema or optic neuritis due to multiple sclerosis. Since they are not present in normal eyes, they almost always signify an abnormality. RNFL evaluation is especially helpful for early glaucoma diagnosis and in glaucoma eyes with small optic disks. In advanced optic nerve atrophy, other examination techniques, such as perimetry, may be more helpful for following optic nerve damage. Considering its great importance in the assessment of optic nerve anomalies and diseases and taking into account the feasibility of its ophthalmoscopic evaluation using green light, the retinal nerve fiber layer should be examined during any routine ophthalmoscopy.     

Reproducibility of topometric data with a scanning laser ophthalmoscope in rabbits

The Heidelberg Retina Tomograph is a confocal scanning laser ophthalmoscope which obtains three-dimensional images of the optic nerve head and the retina in the human eye. Because of its potential investigative uses in experimental animal models of glaucoma, we examined its variability with optic nerve head measurements in the rabbit eye. Three topographic images, recorded on different days, were acquired from the right eye of 5 New Zealand white rabbits over 3 weeks. To estimate the lowest possible variability, a second series of three images was recorded in a single setting without displacement of the rabbits. The average coefficient of variability (standard deviation/mean) for the estimate of the optic nerve head cup volume (volume below surface) was 11.1% in the independent series and was decreased to 3.0% in the sequentially recorded series without displacement of the rabbits (P < 0.001). These values indicate a comparable variability for the estimate of the optic nerve head cup volume in the rabbit compared with those reported for the human eye. This variability is considerably decreased by maximally standardizing the image acquisition position, suggesting that variability largely depends on the alignment between the subject and the laser-scanner.     

HTLV-I associated myelopathy with macular degeneration

The authors report a case of macular involvement in a patient with HTLV-I associated myelopathy (HAM). The patient was a 10-year-old girl who noticed sudden decreased vision in her right eye in November 1987. The corrected visual acuity was 0.01 in the right eye and 1.0 in the left eye. Fundus examination of the right eye disclosed mild optic disc pallor. The macula appeared to have pigmentary mottling with superficial irregular retinal reflex that was three disc diameters in size. Fluorescein angiography revealed a discoid hypofluorescent area in the macula, surrounded by mottled hyperfluorescent areas. She had no contributory family history of ocular disease, but had a history of blood transfusion during an operation for patent ductus arteriosus and ventricular septal defect at the age of 8 months. In November 1990, she developed gait disturbance due to spastic paraparesis and was admitted to our hospital. Antibodies to HTLV-I were markedly elevated in serum (titer, 1:8192) and in cerebrospinal fluid (titer, 1: 1024). She was diagnosed as HAM. Two months later, she developed encephalopathy and bilateral optic disc atrophy.     

Laser photocoagulation for macular soft drusen. Updated results

PURPOSE: To update the results of a study on the disappearance of macular soft drusen after laser treatment in the natural evolution of age-related macular degeneration. METHODS: A total of 46 patients with confluent soft drusen and pigmentary changes were studied prospectively. Group 1 was composed of 30 patients with bilateral drusen; the authors randomly assigned one eye of each patient for treatment and the fellow eye for the control. In 16 patients a choroidal neovascular membrane was present in one eye, and treatment was applied to the fellow eye (group 2). Argon green laser treatment was applied directly to the soft drusen in the temporal macula. RESULTS: All treated drusen disappeared in a mean of 3.5 months after treatment, and untreated drusen disappeared in all but three patients in an average of 8.5 months. After an average period of 3 years, only one control eye and none of the treated eyes in group 1 developed a choroidal neovascular membrane (P = 0.500). In group 2, neovascularization occurred in 18% of the patients. The initial improvement in Snellen acuity after subfoveal drusen disappearance diminished as a consequence of cataract progression. CONCLUSIONS: Although no definitive conclusions should be made because of the small number of patients studied, results seem to show that this treatment does not reduce the risk of choroidal neovascularization in the treated eye of patients with a history of exudative disease in the fellow eye. It may be effective in patients with high-risk bilateral soft drusen, that is, in less advanced stages of the disease.     

Nerve fiber layer defects with normal visual fields. Do normal optic disc and normal visual field indicate absence of glaucomatous abnormality?

PURPOSE: When the optic disc has normal appearance with no abnormalities in routine automated perimetry, the subject is not considered to have glaucoma. The purpose of this study is to show how such patients may have localized retinal nerve fiber layer defects with corresponding functional abnormality. METHODS: The authors selected eight eyes of eight patients who had a localized retinal nerve fiber layer defect extending within a few degrees from fovea but in whom the optic disc appearance and Humphrey 30-2 visual fields were normal. Of the eight patients, three had positive family history of glaucoma, two had suspected retinal nerve fiber layer abnormality in routine eye examination, two had increased intraocular pressure (IOP), and one had advanced low-tension glaucoma in one eye with a normal fellow eye. The authors examined the central 10 degrees visual field with 1 degree resolution using Humphrey perimeter and the Ring and Centring programs of the high-pass resolution perimeter. RESULTS: A central field defect corresponding to retinal nerve fiber layer defect was found in six of eight patients: in both 10 degrees Humphrey field and Centring programs (2 eyes), in Humphrey only (2 eyes), and in Centring only (2 eyes). CONCLUSION: The results indicate that retinal nerve fiber layer photographs are helpful in diagnosing glaucoma because early glaucomatous abnormalities cannot be excluded without nerve fiber layer photography. Currently available routine perimetric examination programs do not always detect very early functional damage.     

Aqueous flare in retinitis pigmentosa

BACKGROUND: The purpose of this study was to quantify blood-ocular barrier impairment by measuring aqueous flare in retinitis pigmentosa (RP) and to search for clinical correlations. METHODS: Forty-nine patients (94 eyes) with RP and 85 normal controls were examined. Aqueous flare was quantified with the noninvasive laser flare-cell meter (FC-1000, Kowa, Japan). Degrees of cystoid macular edema (CME), vitreous pigment dusting (VPD), intraretinal migration of retinal pigment epithelium, and waxy pallor of the optic nerve head were determined semiquantitatively by biomicroscopy. Data were analyzed using the t-test the Mann-Whitney U-test, the chi-squared test and regression analysis by taking into account the dependency of data from two eyes of the same patients. RESULTS: Aqueous flare (photon counts/ms) was significantly higher in RP (mean 10.11 +/- 3.53) than in normals (3.89 +/- 0.94; P < 0.001). Clinically significant CME was present in 26% of eyes with RP, being significantly more frequent in autosomal dominant RP (11 of 16 eyes, 69%) than in other variants (17%; P < 0.005). Multivariate analysis revealed that CME was most strongly associated with flare values (r = 0.84), P < 0.01), whereas--after adjusting for CME--correlations between aqueous flare and other clinical findings did not reach significance. CONCLUSION: RP eyes show increased aqueous flare values, indicating impairment of blood-occur barriers. This appears to be associated with CME and with autosomal dominant RP.     

Reduction in the cumulative incidence rate of cervical cancer by one life time selective screening

PATIENT: A 35-year-old man had suffered from leukemia since September 1990. A transplantation of bone marrow was carried out in February 1994. He developed a graft-versus-host disease in November 1995. In December 1995 a keratoplasty was necessary because of a perforated corneal ulcer. 17 days later the patient noted a complete loss of vision, first in the left and one day later in the right eye. The optic nerve head was white and the retina looked ischaemic like in central retinal artery occlusion. A hypodensic area was found in the frontal brain reaching up to the optic chiasm in computer tomography. Inspite of intensive treatment the immunosuppressed patient died 4 days after he had become blind. Autopsy showed a mycotic infiltration by mucormycosis of the brain and the right optic nerve sheath. This human- pathogenic fungal infection belongs to the group of mould as well as aspergillus. CONCLUSION: Mycosis should be considered in the differential diagnosis of acute visual loss in immunosuppressed patients.